NeuropathologyPub Date : 2025-03-13DOI: 10.1111/neup.70004
Shelley L Forrest, Nusrat Sadia, Mozhgan Khodadadi, Charles Tator, Robin Green, Maria Carmela Tartaglia, Gabor G Kovacs
{"title":"Unprecedented Combination of Rare Degenerative Pathologies in an Octogenarian Ex-Football Player.","authors":"Shelley L Forrest, Nusrat Sadia, Mozhgan Khodadadi, Charles Tator, Robin Green, Maria Carmela Tartaglia, Gabor G Kovacs","doi":"10.1111/neup.70004","DOIUrl":"https://doi.org/10.1111/neup.70004","url":null,"abstract":"<p><p>A 79-year-old former professional football player presented with language deficits and cognitive changes. A year later, he had difficulty completing sentences, and 3 years after onset, was reduced to one-word answers. He developed severe apathy and agitation, and became more impulsive. He eventually became mute and had difficulty with walking and balance. The patient had mild repetitive head injury while playing football and three concussions. Magnetic resonance imaging revealed left > right frontotemporal atrophy. Duration of illness was 6 years. Neuropathology revealed an unexpected number and diversity of degenerative pathologies, including chronic traumatic encephalopathy (CTE, high level), high level Alzheimer's disease neuropathologic change (A3B3C3), limbic Lewy body disease, cerebral amyloid angiopathy (type 2), argyrophilic grain disease (Stage 2), and neuronal intranuclear hyaline inclusion body disease. In addition, there was selective and asymmetric involvement of the corticospinal tract with globular oligodendroglial tau pathology corresponding to globular glial tauopathy (Type II). The patchy and irregular accentuation of cortical tau pathology, particularly in the depths of sulci and accumulation around blood vessels, allows the diagnosis of CTE-neuropathologic change. This diagnosis correlated with the past medical history of multiple concussions. In addition, the patient had an unprecedented number and combination of additional degenerative pathologies, including those that are rare, and how they contributed to the clinical symptoms is difficult to interpret. Globular glial tauopathy Type II is a rare disorder that has been mostly reported in association with progressive supranuclear gaze palsy, and these observations support the notion that globular glial tauopathy Type II is an independent entity with isolated corticospinal tract involvement. These observations highlight that rare disorders can occur in the same individual and be overlooked, especially when there is more obvious pathology. It is essential for neuropathologists to consider an extensive array of neuropathological examinations when assessing patients with neurodegenerative disorders.</p>","PeriodicalId":19204,"journal":{"name":"Neuropathology","volume":" ","pages":""},"PeriodicalIF":1.3,"publicationDate":"2025-03-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143625494","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
NeuropathologyPub Date : 2025-03-05DOI: 10.1111/neup.70003
Ryan K Shahidehpour, Peter T Nelson, Sukanya Srinivasan, Zhong Yu, Adam D Bachstetter
{"title":"Assessing Co-Localization of ITM2B With Alzheimer's Disease and Limbic-Predominant Age-Related TDP-43 Encephalopathy Neuropathologic Changes.","authors":"Ryan K Shahidehpour, Peter T Nelson, Sukanya Srinivasan, Zhong Yu, Adam D Bachstetter","doi":"10.1111/neup.70003","DOIUrl":"10.1111/neup.70003","url":null,"abstract":"<p><p>Mutations in the Integral membrane protein 2B (ITM2B) gene are linked to the development of familial British and Danish dementias, two relatively early-onset dementia disorders known also to be associated with Tau neurofibrillary tangles (NFTs). However, to date, the involvement of ITM2B in limbic-predominant age-related TDP-43 encephalopathy neuropathologic changes (LATE-NC) remains unclear. To address this question, we used brain samples from the University of Kentucky Alzheimer's Disease Research Center community-based autopsy cohort. We investigated the patterns and co-localizations of ITM2B immunohistochemistry in subiculum, CA1, CA2, CA3 and dentate gyrus of the hippocampus from brains with Alzheimer's disease neuropathologic changes (ADNC), LATE-NC, and comorbid ADNC+LATE-NC, as well as low-pathology controls (n = 4 per disease state). There was frequent co-localization between ITM2B protein and intracellular Tau pathology in ADNC; however, there was a far weaker rate of co-localization between ITM2B and TDP-43 pathology. There also was, as previously described, an association between ITM2B immunostaining and neuritic-appearing amyloid plaques. Additionally, co-localization of intracellular ITM2B pathology with Thioflavin-S in NFTs suggested a potential role for ITM2B in marking neurons undergoing transition from relatively healthy (early NFT-bearing cells) to more severely affected (later NFT-bearing) cellular disease states. This study indicates that ITM2B has a relatively specific pattern of involvement in Tau-related neurodegeneration and in neuritic amyloid plaques, while implying minimal, if any, role for ITM2B in the synergistic relationship between Tau and TDP-43 pathologies.</p>","PeriodicalId":19204,"journal":{"name":"Neuropathology","volume":" ","pages":""},"PeriodicalIF":1.3,"publicationDate":"2025-03-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143557666","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
NeuropathologyPub Date : 2025-02-23DOI: 10.1111/neup.70001
Ayça Erşen Danyeli, Zeynep Hüseyinoğlu, M Memet Özek
{"title":"OTX-2 Expression as a Diagnostic Marker for Choroid Plexus Tumors.","authors":"Ayça Erşen Danyeli, Zeynep Hüseyinoğlu, M Memet Özek","doi":"10.1111/neup.70001","DOIUrl":"https://doi.org/10.1111/neup.70001","url":null,"abstract":"<p><p>Choroid plexus tumors are neuroepithelium-derived tumors arising in the ventricles of the central nervous system. They are commonly seen in childhood and correspond to low rates in all central nervous system tumors. Due to their rareness and similar histomorphologic features to other tumors, their diagnosis might be challenging. Here, we used the OTX-2 antibody to evaluate the diagnostic role of OTX-2 expression in choroid plexus tumors. We performed a retrospective review of 34 patients operated for choroid plexus tumors in our center between 2011 and 2023. Additionally, as different tumor types are also arising in the ventricles, we selected five cases each of AT/RT, germ cell tumor, ependymoma, and metastatic adenocarcinoma from the pathology archive. Immunohistochemistry conditions were adjusted for each specific antibody based on the manufacturers' recommendations for concentrations and antigen retrieval/blocking. OTX-2, S-100, and transthyretin antibody staining was performed on sections from each case.</p>","PeriodicalId":19204,"journal":{"name":"Neuropathology","volume":" ","pages":""},"PeriodicalIF":1.3,"publicationDate":"2025-02-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143483515","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
NeuropathologyPub Date : 2025-02-20DOI: 10.1111/neup.70000
Hossam Youssef, Rodolfo G Gatto, Nha Trang Thu Pham, David Jones, Ronald C Petersen, Mary M Machulda, Jennifer L Whitwell, Keith A Josephs
{"title":"Multiple Neuropathologies Underly Hippocampal Subfield Atrophy in a Case With a Slowly Progressive Amnestic Syndrome: Challenging the Notion of Pure LATE-NC.","authors":"Hossam Youssef, Rodolfo G Gatto, Nha Trang Thu Pham, David Jones, Ronald C Petersen, Mary M Machulda, Jennifer L Whitwell, Keith A Josephs","doi":"10.1111/neup.70000","DOIUrl":"https://doi.org/10.1111/neup.70000","url":null,"abstract":"<p><p>Alzheimer's disease (AD) is the leading cause of dementia in the elderly, marked by abnormal protein buildup (beta-amyloid and tau) resulting in neuronal loss, especially in the medial temporal lobe and other limbic regions. The presence of transactive response DNA binding protein 43 (TDP-43) immunoreactive inclusions in medial temporal lobe regions has also been associated with neuroimaging changes in limbic regions. It has been proposed that hypometabolism in limbic regions on [<sup>18</sup>F] fluorodeoxyglucose positron emission tomography (FDG-PET) in a patient with a slowly evolving amnestic syndrome may be a signature of the presence of TDP-43. In this context, we observed an 86-year-old Caucasian female with dementia characterized by a slowly evolving amnestic syndrome, along with focal medial temporal atrophy evident on MRI and hypometabolism in limbic regions on FDG-PET. The patient subsequently died and underwent an autopsy. We performed detailed neuroimaging and digital neuropathological analyses of the hippocampal subfields to better understand the relationship between clinico-imaging findings and histopathology. In addition to TDP-43, we identified three other pathological processes in the medial temporal lobe: sequestosome-1/p62, argyrophilic grain disease (AGD), and primary age-related tauopathy (PART). Hippocampal subfield volumes and rates of atrophy were no different from those of matched healthy controls, except for the atrophy rate in cornu ammonis 1 (CA1). Digital histopathology revealed the relative highest burden of pathology for p62, followed by TDP-43, AGD, and PART in CA1. Multiple pathological processes appear to have contributed to the hippocampal atrophy and hypometabolism in our patient with a slowly progressive amnestic syndrome.</p>","PeriodicalId":19204,"journal":{"name":"Neuropathology","volume":" ","pages":""},"PeriodicalIF":1.3,"publicationDate":"2025-02-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143458754","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
NeuropathologyPub Date : 2025-02-19DOI: 10.1111/neup.70002
Charles Champeaux Depond, Philippe Metellus, Emmanuel Gross, Romain Appay
{"title":"Vanishing Contrast Enhancement of a Diffuse Midline Glioma.","authors":"Charles Champeaux Depond, Philippe Metellus, Emmanuel Gross, Romain Appay","doi":"10.1111/neup.70002","DOIUrl":"https://doi.org/10.1111/neup.70002","url":null,"abstract":"<p><p>Diffuse midline glioma, is a highly aggressive deep-seated glioma whose diagnosis must be confirmed through histopathological analysis of stereotactic biopsies. Hemorrhagic complications after intracranial biopsies may occur, potentially leading to severe neurological sequelae or significantly altering the outcome. A 55-year old male with no significant medical history presented to the local emergency department with 4 days of diplopia. A magnetic resonance imaging (MRI) confirmed the presence of tumor whose characteristics were highly suggestive of a high-grade infiltrating causing blocked hydrocephalus. As no safe resection was achievable, a third ventriculostomy followed by an endoscopic biopsy of the tumor was performed. Unfortunately, the procedure was complicated by an massive intraventricular bleeding of the tumor and, the tiny tumor specimens collected were not contributive. Fortunately, the patient survived and, his clinical state slowly improved. Follow up MRIs depicted a progressive regression of the tumor. As such a wait and see policy was preferred over a chemoradiotherapy. Ultimately, the gadolinium enhancement totally vanished. Unfortunately, 27 months after the initial presentation, he presented with a quick neurological worsening with severe hemiparesis and seizures for which cerebral imaging showed a malignant-looking deep-situated unresectable brain tumor. A second biopsy was performed without any specific complication. The histopathological examination of the tumor revealed a high-grade glial tumor characterized by hypercellularity, marked atypia, mitosis, microvascular proliferation, and areas of necrosis with positive H3 p.K28M nuclear staining in combination with the loss of nuclear H3 p.K28me3. He was referred for best supportive care and, died 29.6 months after the initial presentation. To our knowledge, this is the first report of the gadolinium enhancement of a diffuse midline glioma H3 K27-altered.</p>","PeriodicalId":19204,"journal":{"name":"Neuropathology","volume":" ","pages":""},"PeriodicalIF":1.3,"publicationDate":"2025-02-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143458757","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"An autopsy report of a long-survival case of familial amyotrophic lateral sclerosis with SOD1 G93S gene mutation: Lack of SOD1-positive inclusion in the remaining neurons.","authors":"Asuka Funai, Kentaro Hayashi, Akihiro Kawata, Yuki Nakayama, Chiharu Matsuda, Michiko Haraguchi, Kazushi Takahashi, Takashi Komori","doi":"10.1111/neup.13004","DOIUrl":"10.1111/neup.13004","url":null,"abstract":"<p><p>We describe the case of a 70-year-old Japanese man with familial amyotrophic lateral sclerosis (fALS) associated with a p.Gly93Ser mutation in the copper/zinc superoxide dismutase (SOD1) gene. This mutation is one of the relatively rare SOD1 mutations, with only one previous autopsy report, and is known for its longer disease duration. As previously reported, the patient had weakness in the lower limbs at age 33, followed by dysphagia, dysesthesia in the lower limbs, and autonomic dysfunction. He required mechanical ventilation at age 44 and died of acute pancreatitis at age 70. Neuropathologically, multisystem degeneration was observed beyond lesions typical of familial ALS with posterior column involvement. In addition, there was no SOD1-positive inclusion in the remaining motor neurons. The absence of SOD1-positive inclusion is a rare feature observed predominantly in long survival cases with SOD1 gene mutations. We hypothesize that the considerably lower amount of abnormal SOD1 protein in the motor neuron cells might explain our patient's extraordinarily long clinical course.</p>","PeriodicalId":19204,"journal":{"name":"Neuropathology","volume":" ","pages":"60-65"},"PeriodicalIF":1.3,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142308225","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
NeuropathologyPub Date : 2025-02-01Epub Date: 2024-09-03DOI: 10.1111/neup.13002
Yuting Luo, Pingling Wang, Qinru Zhan, Jiao Luo, Baohong Luo
{"title":"ALK-positive histiocytosis: Report of a rare case with exclusive involvement of the central nervous system in an adult woman.","authors":"Yuting Luo, Pingling Wang, Qinru Zhan, Jiao Luo, Baohong Luo","doi":"10.1111/neup.13002","DOIUrl":"10.1111/neup.13002","url":null,"abstract":"<p><p>ALK-positive histiocytosis is a rare histiocytic disease characterized by ALK positivity. It was first described in 2008 as a systemic disease in infants. The disease often shows positivity for CD68 and CD163 on immunohistochemistry, and genomic analysis frequently reveals KIF5B::ALK fusions. ALK-positive histiocytosis typically follows an indolent course and has a promising prognosis, with conventional treatments usually being effective. Here, we report a rare case of ALK-positive histiocytosis with exclusive involvement of the central nervous system in a 33-year-old Asian adult woman. Although cranial MRI suggested a meningioma, immunohistochemical workup showed that the ALK-positive tumor cells expressed macrophage/histiocyte markers such as CD163 and CD68. Additionally, second-generation sequencing revealed a KIF5B::ALK fusion. Our case highlights the importance of the differential diagnosis in adult central nervous system tumors, emphasizing the combination of morphology, immunophenotype, and molecular approach with ALK status evaluation to confirm a diagnosis of ALK-positive histiocytosis. This case also expands the clinicopathologic spectrum of ALK-positive histiocytosis.</p>","PeriodicalId":19204,"journal":{"name":"Neuropathology","volume":" ","pages":"55-59"},"PeriodicalIF":1.3,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142126275","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
NeuropathologyPub Date : 2025-02-01Epub Date: 2024-06-23DOI: 10.1111/neup.12993
Marzieh Babaee, Yalda Nilipour, Sahar Alijanpour, Aida Ghasemi, Mohammad Mehdi Taghdiri, Payam Sarraf, Mohammad Miryounesi, Mahtab Ramezani
{"title":"Phenotypic and genotyping spectrum of two Iranian cases with RBCK1-associated polyglucosan body myopathy.","authors":"Marzieh Babaee, Yalda Nilipour, Sahar Alijanpour, Aida Ghasemi, Mohammad Mehdi Taghdiri, Payam Sarraf, Mohammad Miryounesi, Mahtab Ramezani","doi":"10.1111/neup.12993","DOIUrl":"10.1111/neup.12993","url":null,"abstract":"<p><p>Glycogen storage diseases (GSDs) are a group of metabolic disorders affecting glycogen metabolism, with polyglucosan body myopathy type 1 (PGBM1) being a rare variant linked to RBCK1 gene mutations. Understanding the clinical diversity of PGBM1 aids in better characterization of the disease. Two unrelated Iranian families with individuals exhibiting progressive muscle weakness underwent clinical evaluations, genetic analysis using whole exome sequencing (WES), and histopathological examinations of muscle biopsies. In one case, a novel homozygous RBCK1 variant was identified, presenting with isolated myopathy without cardiac or immune involvement. Conversely, the second case harbored a known homozygous RBCK1 variant, displaying a broader phenotype encompassing myopathy, cardiomyopathy, inflammation, and immunodeficiency. Histopathological analyses confirmed characteristic skeletal muscle abnormalities consistent with PGBM1. Our study contributes to the expanding understanding of RBCK1-related diseases, illustrating the spectrum of phenotypic variability associated with distinct RBCK1 variants. These findings underscore the importance of genotype-phenotype correlations in elucidating disease mechanisms and guiding clinical management. Furthermore, the utility of next-generation sequencing techniques in diagnosing complex neurogenetic disorders is emphasized, facilitating precise diagnosis and enabling tailored genetic counseling for affected individuals and their families.</p>","PeriodicalId":19204,"journal":{"name":"Neuropathology","volume":" ","pages":"48-54"},"PeriodicalIF":1.3,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141458400","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"An autopsy case of type A FTLD-TDP with a GRN mutation presenting with the logopenic variant of primary progressive aphasia at onset and with corticobasal syndrome subsequently.","authors":"Takafumi Tomenaga, Shinobu Minatani, Hiroto Namba, Akitoshi Takeda, Takahito Yoshizaki, Joji Kawabe, Nazere Keyoumu, Hiroyuki Morino, Makoto Higuchi, Tomoyasu Matsubara, Hiroyuki Hatsuta, Masato Hasegawa, Shigeo Murayama, Yoshiaki Itoh","doi":"10.1111/neup.12980","DOIUrl":"10.1111/neup.12980","url":null,"abstract":"<p><p>A 68-year-old woman presented with difficulty finding words and writing characters. Neurological examination led to clinical diagnosis at onset of the logopenic variant of primary progressive aphasia accompanied with ideomotor apraxia, visuospatial agnosia on the right, and Gerstmann syndrome. Bradykinesia and rigidity on the right with shuffling gait developed after one year. Treatment with L-dopa had no effect. The patient was diagnosed with corticobasal syndrome (CBS). Brain magnetic resonance imaging revealed diffuse cortical atrophy dominantly on the left, especially in the temporal, parietal, and occipital lobes. Positron emission tomography did not reveal any significant accumulation of amyloid β or tau protein. She died five years later. Neuropathological examination revealed diffuse cortical atrophy with severe neuronal loss and fibrous gliosis in the cortex. Neuronal cytoplasmic inclusions, short dystrophic neurites, and, most notably, neuronal intranuclear inclusions, all immunoreactive for phosphorylated TDP-43, were observed. Western blotting revealed a full length and fragments of phosphorylated TDP-43 at 45 and 23 kDa, respectively, confirming the pathological diagnosis of type A FTLD-TDP. Whole exome sequencing revealed a pathogenic mutation in GRN (c.87dupC). FTLD-TDP should be included in the differential diagnosis of CBS.</p>","PeriodicalId":19204,"journal":{"name":"Neuropathology","volume":" ","pages":"38-47"},"PeriodicalIF":1.3,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140876914","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
NeuropathologyPub Date : 2025-02-01Epub Date: 2024-09-23DOI: 10.1111/neup.13003
Daniele Colombo, Laura Falasca, Francesca Monardo, Mario D'Ambrosio, Arianna Di Napoli, Antonio Salerno, Franca Del Nonno, Giovanna Comanducci
{"title":"Neurolymphomatosis mimicking a Guillain-Barré syndrome triggered by COVID-19 vaccination.","authors":"Daniele Colombo, Laura Falasca, Francesca Monardo, Mario D'Ambrosio, Arianna Di Napoli, Antonio Salerno, Franca Del Nonno, Giovanna Comanducci","doi":"10.1111/neup.13003","DOIUrl":"10.1111/neup.13003","url":null,"abstract":"<p><p>Guillain-Barré syndrome (GBS) is an acute disorder of the peripheral nervous system, causing flaccid paralysis, areflexia, and variable sensory involvement. Proximal as well distal muscles of the limbs can be involved, and in most severe and advanced cases progresses to respiratory failure and death. GBS is considered an autoimmune disease, and at the basis of the attack at the peripheral nervous system different mechanisms have been recognized, in particular viral infections or other immune stimulations. Cranial nerve involvement in patients with diffuse large B-cell lymphoma (DLBCL) and primary central nervous system lymphoma are rare conditions that could present with similar clinical features. Here we present a case of a 36-year-old man hospitalized for acute polyradiculoneuritis of the cranial nerves and lumbar roots that arose a 14 days after severe acute respiratory syndrome COVID-19 2 (Sars-CoV-2) vaccination. Most of the main criteria for the diagnosis of GBS were met, including clinical and electrophysiological criteria. Albuminocytologic dissociation and high protein level in cerebrospinal fluid were also found. Therefore, the patient was treated with a cycle of intravenous immunoglobulin (IVIG) with notable improvement of symptoms and gradual recovery of motility. A five months later, following SARS-CoV-2 infection, the patient presented with worsening of neurological symptoms and was readmitted to the hospital. He underwent instrumental tests again and was treated with repeated cycles of IVIG and then with a cycle of plasmapheresis without any improvement. In the following 10 days he developed very serious conditions; he was transferred to intensive care unit and deceased after 6 days. The cause of the neurological syndrome was determined only after autoptic analysis, which revealed the presence of primary peripheral nervous system (PNS) DLBCL. The reported case highlights that GBS-like presentation always requires a careful differential diagnosis, and physicians should also consider the possibility of an occult cancer.</p>","PeriodicalId":19204,"journal":{"name":"Neuropathology","volume":" ","pages":"76-82"},"PeriodicalIF":1.3,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11787998/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142292212","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}